Purpose: To evaluate whether long-term remission of ocular cicatricial pemphigoid (OCP) after withdrawal of immunomodulatory therapy (IMT) is possible.
Methods: A total of 34 of 464 presenting patients (66 eyes) with biopsy-proven OCP in long-term remission off IMT were identified after finishing a 2-year IMT regimen without active disease (2005-2015). Long-term remission off IMT for OCP was defined as patients withdrawn from IMT ≥1 year lacking clinically detectable progressive scarring according to Foster staging and subjective assessment.
Results: All 34 patients achieved ≥1 year of clinical remission without IMT following 2 years IMT lacking active disease. Mean onset age of OCP was 67.0 years, and median follow-up time was 63.4 months. Mean duration between OCP onset and IMT initiation was 29.5 months, with a mean sustained remission time of 36.0 months off IMT. The mean duration of IMT prior to remission off IMT was 34.8 months (median 32 months, IQR 27-39.5 months). Commonly, methotrexate was used prior to OCP remission (19 patients; 55.9%). Two patients experienced mild flare-up postremission off IMT at months 25 and 37 and a course of topical steroid appeared to resolve the inflammation. Another patient had active inflammation at last office visit 5 years after discontinuation of IMT and will restart IMT.
Conclusions: Long-term remission for OCP off IMT may be achieved after stepladder IMT is implemented and withdrawn. Longer follow-up and more sensitive measures of scarring and inflammation are needed to generate a consensus on the definition of complete remission and on cessation of systemic IMT for OCP.
Keywords: Immunomodulatory therapy; Ocular cicatricial pemphigoid; Remission.